[HTML][HTML] Expression of TDP-43 C-terminal fragments in vitro recapitulates pathological features of TDP-43 proteinopathies
LM Igaz, LK Kwong, A Chen-Plotkin, MJ Winton… - Journal of Biological …, 2009 - ASBMB
The disease protein in frontotemporal lobar degeneration with ubiquitin-positive inclusions
(FTLD-U) and amyotrophic lateral sclerosis (ALS) was identified recently as the TDP-43
(TAR DNA-binding protein 43), thereby providing a molecular link between these two
disorders. In FTLD-U and ALS, TDP-43 is redistributed from its normal nuclear localization to
form cytoplasmic insoluble aggregates. Moreover, pathological TDP-43 is abnormally
ubiquitinated, hyperphosphorylated, and N-terminally cleaved to generate C-terminal …
(FTLD-U) and amyotrophic lateral sclerosis (ALS) was identified recently as the TDP-43
(TAR DNA-binding protein 43), thereby providing a molecular link between these two
disorders. In FTLD-U and ALS, TDP-43 is redistributed from its normal nuclear localization to
form cytoplasmic insoluble aggregates. Moreover, pathological TDP-43 is abnormally
ubiquitinated, hyperphosphorylated, and N-terminally cleaved to generate C-terminal …